Endothelin and Systemic Scleroderma |
| Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer |
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Endothelin and Systemic Scleroderma |
| Endothelin and Systemic Scleroderma. Endothelin is a protein that helps to regulate contraction and relaxation of the smooth muscle found in the walls of blood vessels and internal organs (except the heart). An overproduction of endothelin, whether by illness or medication, can cause blood vessels to constrict and blood pressure to increase. In scleroderma, it can cause pulmonary arterial hypertension (PAH). ISN. (Also see: Causes of Scleroderma, What is Scleroderma?, Types of Scleroderma, and Systemic Sclerosis) |
| Endothelin. Endothelins are protein that constrict blood vessels and raise blood pressure. Among the strongest vasoconstrictors known, endothelins are implicated in vascular diseases of several organ systems, including the heart, general circulation and brain. Answers.com. |
| Endothelial dysfunction in pulmonary hypertension (PAH). We review the evidence supporting a central role for endothelial dysfunction in the pathogenesis of PAH as a result of genetic and environmental influences, and extend this concept to include the critical balance between pathways for endothelial growth and survival. Dominique Yelle. (Springerlink) Progress in Inflammation Research, 2010, 81-102. (Also see: PAH) |
| Gene expression profiling in circulating endothelial cells from systemic sclerosis patients shows an altered control of apoptosis and angiogenesis that is modified by Iloprost infusion. We report here that circulating endothelial cells in patients with Systemic Sclerosis show an altered expression of genes involved in the control of apoptosis and angiogenesis. Moreover we describe that Iloprost infusion has a strong effect on endothelial cells and progenitors since it is able to modulate both their number and their gene expression profile. Elisa Tinazzi. Arthritis Research & Therapy 2010, 12:R131. (Also see: Scleroderma Treatments) |
| Capillary dimension measured by computer-based digitalized image correlated with plasma endothelin-1 (ET-1) levels in patients with systemic sclerosis (SSc). The results suggest that computer-based microscopic analysis of nailfold capillaroscopy is a useful method that potentially provides information on organ involvement and plasma ET-1. Capillary dimension maybe a powerful parameter possibly applicable in outpatient clinic for assessing SSc patients. Hyun-Sook Kim J Rheum. (SpringerLink) Clinical Rheumatology. Volume 29, Number 3 / March, 2010. (Also see: Capillaroscopy) |
| New promising therapy against systemic sclerosis. There are three leading actors in the genesis of the diseases. First, the fibroblasts, cells producing connective tissue of all apparatuses, which are the responsible of the production of the tissue- and skin-hardening proteins. Secondly, the endothelial cells, the ones coating the interior part of the vessels and that provoke very dangerous occlusions. Finally, the immune system cells, which are exactly the ones causing an excessive response, that provoke the autoimmune disease. Gianfranco Ferraccioli. EurekAlert! 06/14/10. (Also see: Fibroblasts) |
| Endothelial Fli1 deficiency impairs vascular homeostasis: a role in scleroderma (SSc) vasculopathy. Friend leukemia integration factor 1 (Fli1), an important regulator of immune function and collagen fibrillogenesis, is expressed at reduced levels in endothelial cells in affected skin of patients with SSc. We show that Fli1 regulates expression of genes involved in maintaining vascular homeostasis including VE-cadherin, platelet endothelial cell adhesion molecule 1, type IV collagen, matrix metalloproteinase 9, platelet-derived growth factor B, and S1P(1) receptor. Y Asano.(PubMed) Am J Pathol. 2010 Apr;176(4):1983-98. (Also see: Skin Fibrosis) |
| International conference on endothelin (ET). Identified in 1988, ET is produced by most tissues in the body. The protein is highly concentrated in the brain, lungs, kidney, heart, blood vessels and even in some cancer cells. ET is also the most potent vasoconstrictor (causing the blood vessels to constrict) known. Donna Krupa. EurekAlert. 09/10/09. |
| Raynaud's Phenomenon and Plasma Endothelin (ET-1): Correlations with Capillaroscopic Patterns in Systemic Sclerosis (SSc). Highest ET-1 plasma levels were detected in the more advanced stage of the SSc microangiopathy, namely the late nailfold videocapillaroscopy pattern, characterized by capillary loss and increased tissue fibrosis; this might support the involvement of ET-1 in the progression of the microvascular/fibrotic SSc damage. Alberto Sulli. Journal of Rheumatology. June 2009, 36 (6). (Also see: Raynaud's) |
| When healing turns to scarring: Research reveals why it happens and how to stop it. During tissue repair, specialized cells called myofibroblasts migrate to the wound where they generate the adhesive and tensile forces required for wound closure. Normally, these myofibroblasts then disappear from the wound. But if they persist and continue to make connective tissue, it can become too thick, preventing the organ from functioning properly. EurekAlert! 09/18/08. (Also see: Causes of Scleroderma: Fibroblasts) |
Endothelin and Autoimmunity |
| Elevated platelet-monocyte complexes (PMC) ın patıents wıth psorıatıc arthrıtıs (PsA). Endothelial activation might have a role in the pathogenesis of both psoriasis and PsA. Among parameters of platelet activation, only PMC might play a role in the pathogenesis of PsA. G.E. Pamuk. Platelets. September 14 2009. (Also see: Psoriasis) |
Endothelin Receptor Antagonist (ETRA) |
| Endothelin Receptor Antagonists (ETRA). Endothelin receptor antagonists are a new class of drugs for the treatment of a number of major diseases, including pulmonary arterial hypertension. Endothelin receptor antagonists block endothelin receptors, thereby limiting harmful excess endothelin in the blood vessels. ETRAs include Letairis™ (Ambrisentan) and Tracleer® (Bosentan). PHCentral. August 2009. |
| Improved endothelial function after endothelin receptor blockade in patients with systemic sclerosis (SSc). The results of this study suggest novel vasculoprotective effects of ET(A) receptor antagonism and support further exploration of strategies that target the endothelin 1 pathway in SSc. C. Cardillo. (PubMed) Arthritis Rheum. June 2009 (Also see: Vascular Involvement) |
| Simvastatin Reduces Endothelial Activation and Damage But Is Partially Ineffective in Inducing Endothelial Repair in Systemic Sclerosis (SSc). Treatment with simvastatin results in rapid and significant improvement of measures of endothelial activation, suggesting a potential role of statins in the treatment of peripheral vascular disease in SSc. Journal of Rheumatology. June 2008. (Also see: Medications and Raynaud's) |
